18^th International meeting of the World Federation of Neurology Research Group on Huntington’s Disease 28^th–31^st August 1999 and 13^th International Meeting of the International Huntington Association 28^th August to 2^nd September 1999.

Conferance report by Shirley Elliott-Galloway of the Christchurch HD Assocation

The first part of this conference was held at the Kurhaus Hotel in The Hague Scheveningen, Holland, a beautiful old seaside resort area. After the welcome and official opening joint session the two groups separated and the IHA delegates introduced themselves. There were eighty five delegates and observers present from twenty five countries. This was followed by a presentation by Dr Karen Sirmon (USA) about using IVF for people who had a positive test result to ensure their children would not carry the HD gene before implanting ‘clear’ embryo’s. Morning tea was next followed by Dr Seymour Kessler (USA) who talked about ‘Caring for Carers’ such as training and support groups for caregivers, then a panel session ‘Coping with Caring’, Dr Kessler, Jim Pollard and Dr Ken Barrett. Providing help where needed for caregivers other than professionals is a real problem in many countries as the average number of affected people is only four to seven per 100,000 of population. Suggested strategies were ‘time out’ by using whatever help can be begged, borrowed or stolen from other family members, friends or professionals and by using whatever short term residential care is available.

Following lunch we had a predictive testing update from Professors Aad Tibbenaud Gerry Kieboms of Holland. It is now ten years since testing became available and only about 20% of eligible ‘at risk’ people are taking the test, for many reasons. They have found people have as many reasons for not taking the test as those who do take it. Dr David Crauford (UK) then told us something about medical treatments such as anti-depressants, SSRI’s, Tricyclics, MAOI’s, Prozac, Sodium Valproate, and other low dose Neuroleptics, all of which seem to have varying side effects and are no longer being used as widely as previously although all can be useful in some cases. Some make people drowsy and some seem to cause insomnia, although this can be helped by way of a special duvet. He told us it has holes in it but didn’t have a sample to show. That was the end of what was for me a very long day, but some people went to a concert that night. My suitcase, along with many others, had been left at Heathrow in London so several of us only had the clothes we travelled in to wear for three days, but at last they did turn up again.

Day two began with a research update/future therapies presented by Prof. Anne Young (USA). Although other cells renew themselves constantly, nerve cells don’t .The gene Huntingtin contains 3000 proteins, other genes have only 1000 so there is still a great deal of work to be done analysing their effects. Anne’s team has found that an HD mouse model injected with Tetracyclines reversed damage caused by the HD gene and are very excited about what they are seeing. Her advice is to stay active, positive and happy, to help delay onset and effects of the disease because, her words, ‘we are just so close to beating it’. Her husband, Dr John Penney, died earlier this year and was also on the research team in Boston so was a great loss to all of us.

Then came Dr Ira Shoulson (USA) with current therapies/Huntington’s study group CARE-HD using coenzyme Q10 600mls orally per day and Remacemide, RID-HD using Riluzole. These have been mentioned in our September newsletter. Morning tea was followed by an update on neural transplantation from Dr Marco Peschanski (France) who told us that after two years of a five year study there have been no adverse effects noted. This procedure is used with many safeguards, is still experimental and was described at the previous conference two years ago as well as our newsletter following that conference. Nothing really new at this time a they require five years to assess results. Dr Ken Barrett (UK) then described the role of the psychiatrist and medications used for psychiatric problems , such as Venlafaxine, Sulpiride and Clozopine, which are used as little as possible and only short term. Thank goodness for the lunch break!!

The afternoon session began with another neural transplant update – Capit HD from Dr Anne Rosser (Cambridge UK). They started assessing patients in 1996 when the use of foetal tissue was permitted with full consent and screening. Like all these procedures five years is required to assess results so should all know more in three years time. We than had a joint meeting with the WFN group describing models of care in the UK, USA, Australia, and Holland which included a video of life in a specialist unit in Holland, an overview from Jim Pollard (USA) and a discussion on the above.

Coping with challenging behaviour, also one of the topics, made me more fully aware that the world is really only one country and we are all trying our best to cope with the same things, although larger populations can obviously supply more facilities for specialised care and research. That closed work for the day and as my suitcase had finally arrived I went back to the Badhotel (I think it means bathhouse) to shower and change before being taken by tram to The Hague for a reception by the Mayor, Dr W J Deetman, and the conference dinner at the Pulchri Studio which is a beautiful, very old building now used mainly for art shows.

Day three, the last day of the joint conference, began with ‘Understanding Challenging Behaviour’ from Dr Julie Snowden (UK) based on the CAP-IT test to measure damage to the brain cells and the difficulties the amount of damage causes for both patients and carers. Patience all round is the name of the game. Gerrit Dommerholt (Holland) then told us about progress in international development of associations around the world. I think we had three new countries represented at this conference, one of which was Brazil who with a population of 160 million only formed an association in 1997. Others were Russia, where the average wage is $US50 per month and Japan with a total of 393 known cases (0.72 per 100,000 population). Two Japanese doctors with one young association member sang a beautiful song as part of their presentation. They looked and sounded like teenage schoolgirls, very well presented to a very appreciative audience. We then had ‘Making Stone Soup’ which was an Australian offering and I think it was a skit on involving others to form group efforts.

Lunch came next, then a tourist ride across Holland to the venue for the second part of the conference. Holland is a beautiful country in early September, lovely countryside with lots of water, gardens and wonderful little villages with some very old buildings. The venue is called Landgoed Ehzerwold, not in a town and was originally built as a sanatorium then used for refugees and is now a resort and conference centre. Great place for a non-city person like me.

Morning tea was followed by an overview of the conference from Prof. Raymund Roos (Holland) which I have attempted to give you with the above. He then talked about Juvenile HD, i.e. under twenty years old and very rare, with unclear symptoms which makes it difficult to diagnose. There was then a discussion about systems and support for children and parents which seem to be more or less similar to ours. After lunch Ineke Lonink told us about the Dutch experience as an association which consists of lay people only, founded in 1976 with between 200-250 family members and now with 1400 members. They have a National Office with two staff in the Hague and have funding from the Princess Beatrix foundation and train lay people to teach clinical lessons to professionals. They also have a young peoples committee consisting of 20-45 members and a great deal of other financial support as the travel across Holland and the two days of conference at Ehzerwold, as well as the conference dinner for eight five of us were all paid for by sponsorship.

The rest of the afternoon was the International Huntington’s Assn. business meeting with election of officers to the Board which remains the same with the exception of treasurer, now Ron Livingstone of Scotland. Ralph Walker (Canada) was treasurer for twenty five years and has now been elected Honorary Member of the board in recognition of his contribution to the IHA. Minutes of the last meeting and financial statements were also read, explained and approved. The next international meeting is to be held in August 2001 in Copenhagen, Denmark.

The last morning was about ethical issues, a committee was formed at the business meeting to deal with these, insurance issues, which are still causing some problems, and reports from new associations. Sue Watkin (UK) who is president of the IHA then gave a summary of the conference. In the afternoon I was part of a group which visited Hullenoord Nursing Home where we met several of the HD patients as well as the director and several staff members. Very well laid out and organised as well as in a very nice setting.

I would like to add that as the only NZ delegate at this conference, my third, I felt really privileged to be able to attend on your behalf, to interact with so many caring positive people, both professional and family members like myself, and to be able to pass so much of it on to you. There were 164 members at the WFN meeting, including four from New Zealand, and they all really care about all of us and are working and hoping for a solution just as hard as we are.

Shirley Elliott-Galloway

Christchurch HD Assn Committee Member.